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Left Cardiac Sympathetic Denervation Monotherapy in Patients With Congenital Long QT Syndrome

Niaz, Talha ; Bos, J Martijn ; Sorensen, Katrina B ; Moir, Christopher ; Ackerman, Michael J

Circulation. Arrhythmia and electrophysiology, 2020-12, Vol.13 (12), p.e008830-e008830 [Periódico revisado por pares]

United States

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Título:
Left Cardiac Sympathetic Denervation Monotherapy in Patients With Congenital Long QT Syndrome
Autor: Niaz, Talha ; Bos, J Martijn ; Sorensen, Katrina B ; Moir, Christopher ; Ackerman, Michael J
Assuntos: Adolescent ; Adult ; Child ; Child, Preschool ; Clinical Decision-Making ; Female ; Heart - innervation ; Heart Rate ; Humans ; Long QT Syndrome - congenital ; Long QT Syndrome - diagnosis ; Long QT Syndrome - physiopathology ; Long QT Syndrome - surgery ; Male ; Recurrence ; Retrospective Studies ; Romano-Ward Syndrome - diagnosis ; Romano-Ward Syndrome - genetics ; Romano-Ward Syndrome - physiopathology ; Romano-Ward Syndrome - surgery ; Sympathectomy - adverse effects ; Sympathetic Nervous System - physiopathology ; Sympathetic Nervous System - surgery ; Time Factors ; Treatment Outcome ; Video-Assisted Surgery - adverse effects ; Young Adult
É parte de: Circulation. Arrhythmia and electrophysiology, 2020-12, Vol.13 (12), p.e008830-e008830
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
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Descrição: Videoscopic left cardiac sympathetic denervation (LCSD) is an effective antifibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events while on beta-blockers, LCSD as 1-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our early experience with LCSD monotherapy for carefully selected patients with LQTS. Among the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to identify the patients where the LCSD served as stand-alone, monotherapy. Clinical data on symptomatic status before diagnosis, clinical, and genetic diagnosis, and breakthrough cardiac events after diagnosis were analyzed to determine efficacy of LCSD monotherapy. Overall, 64 of 204 patients (31%) were treated with LCSD alone (37 [58%] female, mean QTc 466±30 ms, 16 [25%] patients were symptomatic before diagnosis with a mean age at diagnosis 17.3±11.8 years, 5 had [8%] ≥1 breakthrough cardiac event after diagnosis, and mean age at LCSD was 21.1±11.4 years). The primary motivation for LCSD monotherapy was an unacceptable quality of life stemming from beta-blocker related side effects (ie, beta-blocker intolerance) in 56/64 patients (88%). The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%). There were no significant LCSD-related surgical complications. With a mean follow-up of 2.7±2.4 years so far, only 3 patients have experienced a nonlethal, post-LCSD breakthrough cardiac event in 180 patient-years. LCSD may be a safe and effective stand-alone therapy for select patients who do not tolerate beta-blockers. However, LCSD is not curative and patient selection will be critical when potentially considering LCSD as monotherapy.
Editor: United States
Idioma: Inglês

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Left Cardiac Sympathetic Denervation Monotherapy in Patients With Congenital Long QT Syndrome

Niaz, Talha ; Bos, J Martijn ; Sorensen, Katrina B ; Moir, Christopher ; Ackerman, Michael J

United States

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